title: [Teratoid/rhabdoid tumor of the central nervous system].
abstract: Teratoid/rhabdoid tumor is a recently introduced infantile brain neoplasm of uncertain origin clinically characterized by a most aggressive course. We describe the case of a 2-year-old boy and a 7-year-old girl, the former presenting with central fever and hemiparesis, while vomiting and headache were symptoms in the latter. A brain tumor in the right fronto-parieto-occipital and the left fronto-medial parenchyma, respectively, were found and removed by surgery. On histology, both lesions consisted of sheets of ovoid cells with prominent cytoplasm, vesicular nuclei and macronucleoli. In the second case, a spindle-cell component and bone metaplasia were also noted. Ultrastructurally, tumor cells contained whorls of intermediate filaments. Immunohistochemistry revealed a polyphenotypic expression profile including positivity for epithelial membrane antigen, vimentin and a-actin. There was no evidence of a germ-cell origin. Chromosomal translocation involving 22q11 was established cytogenetically in the first case. The teratoid/rhabdoid tumor shares many common traits with renal rhabdoid tumors and primitive neuroectodermal tumors as well as germ cell neoplasms; therefore its being an entity is debated.
authors.affiliation: Neuropathologiai Intézet, Neues Allgemeines Krankenhaus Wien.
authors.forename: I
authors.initials: I
authors.lastname: Vajtai
authors.forename: Z
authors.initials: Z
authors.lastname: Varga
mesh_terms: D001932:Brain Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D018335:Rhabdoid Tumor / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D013724:Teratoma / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery
vernacular_title: A központi idegrendszer teratoid/rhabdoid tumora.